Kidney tumor


WHO classification

Last author update: 1 December 2016
Last staff update: 13 September 2022 (update in progress)

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PubMed search: WHO classification[TIAB] (kidney tumors[TIAB] OR renal tumors[TIAB])

Nicole K. Andeen, M.D.
Maria Tretiakova, M.D., Ph.D.
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Table of Contents
Definition / general | WHO (2016)
Cite this page: Andeen NK, Tretiakova M. WHO classification. website. Accessed December 2nd, 2022.
Definition / general
  • This topic is based on the 2016 World Health Organization classification (Moch: WHO Classification of Tumours of the Urinary System and Male Genital Organs, 4th Edition, 2016)
  • We currently plan to update this topic at the end of 2021 based on the new WHO 5th edition
  • Based on cytologic, architectural, immunohistochemical, cytogenetic and molecular characteristics
  • New entities continue to be described based on immunohistochemical and molecular features - some not yet recognized in WHO Classification
  • Use of "granular cell variant" not recommended - may falsely combine a spectrum of tumors with varying clinical significance / treatment: clear cell, chromophobe, unclassified renal cell carcinoma or oncocytoma (Urology 2007;69:452)
  • Diagnosis of "sarcomatoid carcinoma" not recommended; classify as high grade carcinoma of type from which it originated (or unclassified) with sarcomatoid features and percentage of sarcomatoid features (Cancer 1997;80:987)
WHO (2016)
Renal cell tumors
  • Clear cell renal cell carcinoma
  • Multilocular cystic renal neoplasm of low malignant potential
  • Papillary renal cell carcinoma
  • Hereditary leiomyomatosis and renal cell carcinoma associated renal cell carcinoma
  • Chromophobe renal cell carcinoma
  • Collecting duct carcinoma
  • Renal medullary carcinoma
  • MiT family translocation renal cell carcinomas
  • Succinate dehydrogenase deficient renal cell carcinoma
  • Mucinous tubular and spindle cell carcinoma
  • Tubulocystic renal cell carcinoma
  • Acquired cystic disease associated renal cell carcinoma
  • Clear cell papillary renal cell carcinoma
  • Renal cell carcinoma, unclassified
  • Papillary adenoma
  • Oncocytoma

Metanephric tumors
  • Metanephric adenoma
  • Metanephric adenofibroma
  • Metanephric stromal tumor

Nephroblastic and cystic tumors occurring mainly in children
  • Nephrogenic rests
  • Nephroblastoma
  • Cystic partially differentiated nephroblastoma
  • Pediatric cystic nephroma

Mesenchymal tumors

Mesenchymal tumors occurring mainly in children
  • Clear cell sarcoma
  • Rhabdoid tumor
  • Congenital mesoblastic nephroma
  • Ossifying renal tumor of infancy

Mesenchymal tumors occurring mainly in adults
  • Leiomyosarcoma (including renal vein leiomyosarcoma)
  • Angiosarcoma
  • Rhabdomyosarcoma
  • Osteosarcoma
  • Synovial sarcoma
  • Ewing sarcoma
  • Angiomyolipoma
  • Epithelioid angiomyolipoma
  • Leiomyoma
  • Hemangioma
  • Lymphangioma
  • Hemangioblastoma
  • Juxtaglomerular cell tumor
  • Renomedullary interstitial cell tumor
  • Schwannoma
  • Solitary fibrous tumor

Mixed epithelial and stromal tumor family
  • Adult cystic nephroma
  • Mixed epithelial and stromal tumor

Neuroendocrine tumors
  • Well differentiated neuroendocrine tumor
  • Large cell neuroendocrine carcinoma
  • Small cell neuroendocrine carcinoma
  • Paraganglioma

Renal hematopoietic neoplasms

Germ cell tumors

Metastatic tumors

Tumor-like lesions
  • Xanthogranulomatous pyelonephritis
  • IgG4 related disease

Described entities not currently in WHO
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